Pulmonary hypertension (PH) is a disorder with poor prognosis sustained by progressive elevation of pulmonary vascular resistance to blood flow. It is a serious condition for which treatments exist but there is no cure. The main determinants are vasoconstriction, pulmonary vascular remodelling, and in situ thrombosis associated with exuberant cellular proliferation and precapillary arterial bed obstruction. When unrecognized or untreated, PH has an unpredictable but worsening course that leads to cardiorespiratory decompensation. In the past ten years there have been significant improvements in both diagnostic and therapeutic strategies that have led to substantial changes in prognosis. However the number of subjects who over time become unresponsive to maximal medical treatment is still considerable so that surgical interventions are needed as last chance for survival. Lung transplantation (LTx) is a viable therapy for severe symptomatic PH. Even though survival after LTx has improved consistently, the recipients with an underlying diagnosis of PH seem to be affected by a greater 1-year mortality following LTx compared to recipients with other diagnoses. This article discusses the disease mechanisms and the pathophysiological processes involved in the development of PH. It provides some information on current and newer pharmacologic options, and it also considers the physiologic aspects of lung transplantation.
Keywords: Pulmonary arterial hypertension, pulmonary vasodilators, cardiorespiratory functional class, pulmonary, thromboendoarterectomy, lung transplantation, lung graft function